Abstract
Previous work has demostrated marked phospaturia in thallassemia major, resulting in a negative phosphorus balance (Lapatsanis et al.Ped. 58:885,76). Therefore it was decided to study the effects of oral phosphate administration on serum phosphorus (Pi), red cell adenosine triphosphate (ATP) and 2,3-diphosphoglycerate (2,3-DPG) in this desease. Group B of the table which was regularly transfused as was groupA(control) received l,5-2.0g of Pi as phosphate salt daily, p.o.
Hemoglobin and hematocrit were not significantly different in the two roups. Serum Pi was significantly higher in group B compared to that of group A (p<0.01). Mean red cell ATP was higher in group B from that of group A by 41%. The difference was highly significant (p < 0.001). On the contrary there was no difference in the mean red cell 2,3-DPG between the two groups.
The elevation of ATP was an expected and possibly beneficial finding. There is no explanation for the lack or response of 2,3-DPG to the raised serum Pi in group B. Since most of the red cells in these children were donor cells it indicates possibly an abnormality of blood bank blood in 2,3-DPG metabolism.
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Karaklis, A., Lapatsanis, P., Stamoulakatou, S. et al. EFFECTS OF PHOSPHORUS ADMINISTRATION IN THALASSEMIA MAJOR. Pediatr Res 12, 68 (1978). https://doi.org/10.1203/00006450-197801000-00048
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DOI: https://doi.org/10.1203/00006450-197801000-00048